Second harvest of Congenital Heart Surgery Database in Türkiye: Current outcomes.

Background: This second harvest of the Congenital Heart Surgery Database intended to compare current results with international databases. Methods: This retrospective study examined a total of 4007 congenital heart surgery procedures from 15 centers in the Congenital Heart Surgery Database between January 2018 and January 2023. International diagnostic and procedural codes were used for data entry. STAT (Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery) mortality scores and categories were used for comparison of the data. Surgical priority status was modified from American Society of Anesthesiologist guidelines. Centers that sent more than 5 cases to the database were included to the study. Results: Cardiopulmonary bypass and cardioplegic arrest were performed in 2,983 (74.4%) procedures. General risk factors were present in 22.6% of the patients, such as genetic anomaly, syndrome, or prematurity. Overall, 18.9% of the patients had preoperative risk factors (e.g., mechanical ventilation, renal failure, and sepsis). Of the procedures, 610 (15.2%) were performed on neonates, 1,450 (36.2%) on infants, 1,803 (45%) on children, and 144 (3.6%) on adults. The operative timing was elective in 56.5% of the patients, 34.4% were urgent, 8% were emergent, and 1.1% were rescue procedures. Extracorporeal membrane oxygenation support was used in 163 (4%) patients, with a 34.3% survival rate. Overall mortality in this series was 6.7% (n=271). Risk for mortality was higher in patients with general risk factors, such as prematurity, low birth weight neonates, and heterotaxy syndrome. Mortality for patients with preoperative mechanical ventilation was 17.5%. Pulmonary hypertension and preoperative circulatory shock had 11.6% and 10% mortality rates, respectively. Mortality for patients who had no preoperative risk factor was 3.9%. Neonates had the highest mortality rate (20.5%). Intensive care unit and hospital stay time for neonates (median of 17.8 days and 24.8 days, respectively) were also higher than the other age groups. Infants had 6.2% mortality. Hospital mortality was 2.8% for children and 3.5% for adults. Mortality rate was 2.8% for elective cases. Observed mortality rates were higher than expected in the fourth and fifth categories of the STAT system (observed, 14.8% and 51.9%; expected, 9.9% and 23.1%; respectively). Conclusion: For the first time, outcomes of congenital heart surgery in Türkiye could be compared to the current world experience with this multicenter database study. Increased mortality rate of neonatal and complex heart operations could be delineated as areas that need improvement. The Congenital Heart Surgery Database has great potential for quality improvement of congenital heart surgery in Türkiye. In the long term, participation of more centers in the database may allow more accurate risk adjustment.

Successful thrombolysis of a subacute prosthetic valve thrombosis with modified ultra-slow thrombolytic therapy.

Prosthetic valve thrombosis is a serious complication of heart valve surgery. According to the recent studies, thrombolytic therapy is an important alternative therapy to the open heart surgery with successful outcomes. We present a case of a prosthetic mitral valve thrombosis who was successfully treated with modified ultra-slow thrombolytic therapy.

Treating a Patient of Dysfibrinogenemia with Acute Thromboembolism by Rivaroxaban and Cilostazol.

Congenital dysfibrinogenemia is a rare autosomal recessive bleeding disorder, which is characterized by the absence of functional fibrinogen. Patients may have bleeding and paradoxical arterial and venous thrombotic problems from early childhood. The optimal antithrombotic therapy in these patients hasn't been determined yet. In this report we present a dysfibrogenemic patient, who has suffered recurrent arterial thrombosis under aspirin treatment. Intravenous fibrinogen concentrates (fc) along with reduced doses of rivaroxaban (10 mg daily), cilostazol (50 mg bid) and aspirin (100 mg daily) were given as antithrombotic treatment. The pain and the cyanosis clinically recovered within 6 weeks. This is, to our knowledge, the first time in which a new oral anticoagulant, rivaroxaban and cilostazol combination was used in a dysfibrinogenemic patient with thrombotic episodes. We determined the type, the dosage and the duration of antithrombotic treatment according to the clinical progress of the symptoms. Rivaroxaban, cilostazol and fibrinogen concentrate replacement; combination may represent a useful alternative for the antithrombotic treatment in dysfibrinogenemic patients.

Huge Left Atrial Pseudoaneurysm in a 5-Month-Old Baby Presented with Supraventricular Tachycardia.

UNLABELLED: Cardiac pseudoaneurysm is an extremely rare condition in children. It can be congenital or acquired and has been primarily described in the left side of the heart. The congenital form of pseudoaneurysm is thought to be related to muscular dysplasia of the muscular layer of the heart. The acquired form is related to myocardial infarction, infective endocarditis or chest trauma. In this report we described a 5-month-old baby with left atrial pseudoaneurysm who was referred to our hospital due to tachycardia and hemodynamic instability. After diagnosis of left atrial pseudoaneurysm, aneurysmectomy was performed and the left atrial wall was successfully repaired by surgery three days after admission. In conclusion, due to life threatening complications such as dissection, arrhythmias, compression of aneurysm and tromboembolic complications, pseudoaneurysm should be treated by surgery. KEY WORDS: Chest trauma; Left atrial pseudoaneurysm; Supraventricular arrhythmias.

Early initiation of peritoneal dialysis after arterial switch operations in newborn patients.

BACKGROUND AND AIM: We investigated the clinical outcome of early initiated peritoneal dialysis (PD) use in our newborn patients who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) and had routine intraoperative PD catheter implantation. We determined the risk factors for PD, factors associated with prolonged PD, morbidity, and mortality. The aim of the present study was to describe our experience of using PD in this patient cohort. MATERIALS AND METHODS: Eighty two patients who were diagnosed with TGA and TGA-ventricular septal defect (VSD) and who had undergone TGA correction operation in Baskent University, Istanbul Medical Research and Training Hospital between 2007 and 2012 were retrospectively investigated. All the patients were under 30 days old. PD catheters were routinely implanted intraoperatively at the end of the operation. PD was initiated in transient renal insufficiency. In the absence of oliguria and increased creatinine level, PD was established in the presence of one of the following: clinical signs of fluid overload, hyperkalemia (>5 mEq/L), persistent metabolic acidosis, lactate level above 8 mmol/L or low cardiac output syndrome. The patients were divided into two groups according to the need for postoperative PD (PD group and non-PD group). PD was initiated in 32 (39%) patients after the operation, whereas 50 (61%) patients did not need dialysis. The clinical outcomes and perioperative data of the two groups were compared. RESULTS: The demographics in the two groups were similar. Cardiopulmonary bypass time was longer in the PD group [non-PD group, 175.24 ± 32.39 min; PD group, 196.22 ± 44.04 min (p < 0.05)]. Coronary anomaly was found to be higher in the PD group [non-PD group, n = 2 patients (4.0%); PD group, n = 7 patients (21.9%); p < 0.05]. There was more need for PD in TGA + VSD patients [simple TGA patients, n = 14; TGA + VSD patients, n = 18 (p < 0.05)]. PD rate was higher in patients whose sterna were left open at the end of the operation (p < 0.05). The ventilator time [non-PD group, 4.04 ± 1.51 days; PD group, 8.12 ± 5.21 days (p < 0.01)], intensive care unit stay time [non-PD group, 7.98 ± 5.80 days; PD group, 15.93 ± 18.31 days (p < 0.01)], and hospital stay time were significantly longer in the PD group [non-PD group, 14.98 ± 10.14 days; PD group, 22.84 ± 20.87 days (p < 0.01)]. CONCLUSION: We advocate routine implantation of PD catheters to patients with TGA-VSD, coronary artery anomaly, and open sternum in which we have determined high rate of postoperative PD need.

Coronary artery fistula between left coronary artery and coronary sinus in newborn.

Aneurysmal circumflex coronary artery fistula connected to the coronary sinus is a rare clinical entity that usually remains asymptomatic until later in life. The timing of surgical treatment for asymptomatic patients is crucial. The decision to leave or exclude the aneurysmatic coronary artery following ligation of the fistula is controversial. Herein, we report the successful management of a coronary fistula between the circumflex artery and the coronary sinus without using cardiopulmonary bypass during the newborn period.

Delayed sternal closure after pediatric cardiac operations; single center experience: a retrospective study.

BACKGROUND: Delayed sternal closure (DSC) after cardiac surgery is a therapeutic option in the treatment of the severely impaired heart in pediatric cardiac surgery. The results with the technique of DSC over a 4-year period are examined with regard to mortality and morbidity. METHODS: We retrospectively reviewed records of 38 patients who had undergone DSC among 1100 congenital cardiac operations. Indication of DSC, time to sternal closure, pre and post closure cardiopulmonary and metabolic status, mortality, rate of wound and bloodstream infections were recorded. RESULTS: The mean sternal closure time was 2.9 days. The mortality rate was 34.2% (n = 13). Twenty (52.6%) patients required prolonged antibiotic use due to postoperative infection. There was gram negative microorganism predominance. There were 4 (10.5%) patients with postoperative mediastinitis. Postoperative infection rate statistically increased with cardiopulmonary bypass time (CPBT), sternal closure time (SCT) and intensive care unit (ICU) stay time (p = 0.039;p = 0.01;p = 0.012). On the other hand, the mortality rate significantly increased with increased cross clamp time (CCT), SCT, and extracorporeal membrane oxygenation (ECMO) use (p = 0.017; p = 0.026; p = 0.03). Single ventricular physiology was found to be risk factor for mortality in delayed sternal closure (p < 0.007). CONCLUSIONS: Elective DSC does not reduce the morbidity. The prolonged sternal closure time is associated with increased rate of postoperative infection rate; therefore early closure is strongly advocated.

Risk factors for postoperative arrhythmia in patients with physiologic univentricular hearts undergoing Fontan procedure.

OBJECTIVE: Advanced age, dilated right atrium, increased preoperative pulmonary artery pressure, increased right atrial pressure, technique of operation, and poor ventricular function were reported to be risk factors for postoperative arrhythmia. Aim of this study is to determine the risk factor for postoperative arrhythmia after Fontan operation with regard to ventricle dominancy and hemodynamic parameters. METHODS: In this retrospective study, the data of the patients including age, weight, dominant ventricle, type of cardiac anomaly, previous operations, duration of cardiopulmonary bypass (CPB), duration of aortic clamping, cardiac rhythm, pulmonary artery pressure, the Nakata Index, systemic atrioventricular (AV) valve insufficiency were obtained from the hospital records, the echocardiographic files, and cardiac catheterization records. Patients were assigned to dominant left ventricle or dominant right ventricle groups. Statistical analysis was performed using the Mann-Whitney U test. RESULTS: Arrhythmia was observed in 21 (52.5%) patients in the postoperative period. No postoperative arrhythmia was observed in patients with a right atrial pressure of <5 mmHg, whereas postoperative arrhythmias were observed in patients with a right atrial pressure of ≥5 mmHg (p<0.05). When the effects of preoperative and postoperative pulmonary artery pressures on postoperative arrhythmia were evaluated, postoperative arrhythmia was determined in only 2 (12.5%) of 8 patients with a preoperative mean pulmonary artery pressure of ≤9 mmHg, whereas postoperative arrhythmia was observed in 19 (59.3%) of 32 patients with a preoperative pulmonary artery pressure of ≥10 mmHg. A preoperative mean pulmonary artery pressure of >10 mmHg increased the risk of postoperative arrhythmia (p<0.02). Postoperative arrhythmia was determined in 8 (53%) of 15 patients with a dominant right ventricle, and in 13 (52%) of 25 patients with a dominant left ventricle (p>0.05). CONCLUSION: While a preoperative mean pulmonary artery pressure of >10 mmHg and a preoperative right atrial pressure of >5 mmHg were the risk factors for postoperative arrhythmia in patients undergoing Fontan procedure, the right or left ventricular dominance was not a risk factor.

Valve-sparing replacement of the ascending aorta and aortic arch in a child with Loeys-Dietz syndrome.

We describe a successful surgical treatment in a 2.5-year old boy with Loeys-Dietz syndrome, in whom we performed aortic arch and ascending aorta replacement with a valve-sparing operation (VSO) of the aortic root because of significant aortic insufficiency and dilation of the aortic root. We believe that VSO is ideal for treating young patients with aortic root aneurysm with normal or minimally diseased aortic cusps to avoid the disadvantages of prosthetic valve replacements.

Treatment of subaortic stenosis in hearts with single-ventricle physiology.

BACKGROUND: We evaluated the patients who had had a Damus-Kaye-Stansel (DKS) operation for single-ventricular physiology with the aorta originating from a hypoplastic ventricle and the pulmonary artery from the systemic ventricle. METHODS: Seven patients who were operated on between May 2007 and November 2010 were evaluated retrospectively. The patients had been diagnosed with a transposed double-inlet left ventricle and triscuspid atresia, and had been waiting for a Fontan operation. Systemic outflow stenosis was defined echocardiographically as those with a gradient greater than 20 mmHg, and angiographically those with greater than 5 mmHg in the subaortic region. RESULTS: The mean age and weight of the patients was 15 ± 9.7 months and 8 ± 3.3 kg, respectively. The mean gradient between the systemic ventricle and the aorta was 35 ± 25 mmHg. This gradient decreased to 14.3 ± 4 mmHg postoperatively. The early hospital mortality was 14% (one patient). The mean extubation time and mean time in the intensive care unit (ICU) were 13 ± 7.3 hours and 2.2 ± 0.5 days, respectively. The mean follow-up time was 11 ± 2 months. No mortality and semi-lunar valve insufficiency were observed after discharge. CONCLUSIONS: One of the major problems that occur while waiting for a Fontan operation is systemic ventricular hypertrophy and deterioration in the compliance of the ventricle due to systemic ventricular outflow stenosis. When the disadvantages of outflow resection are encountered, a DKS proves to be a good alternative.